Abstract
We herein report a case of Childhood Primary Central Nervous System (CNS) Angiitis. This case consisted of a 14-year-old girl who presented with right-sided weakness, aphasia, and a fever. A Magnetic Resonance Imaging of the brain showed multifocal areas of ischemia. Magnetic Resonance Angiography of the head and neck showed narrowing and irregularities of the left middle cerebral artery and right posterior cerebral artery. Cerebrospinal Fluid studies showed a lymphocytic pleocytosis and brain biopsy revealed leptomeningeal and perivascular inflammation. The epidemiology, presenting symptoms, work-up, pathophysiology, diagnostic criteria, and treatment of Childhood Primary CNS angiitis are discussed. This case serves as a reminder that when pediatric patients present with stroke-like symptoms inflammatory etiologies including Primary CNS Angiitis must be considered and treated appropriately in a time sensitive manner.